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Cumulative incidence plot comparing (A) Ann Arbor stage, (B) calendar year of diagnosis (C) age at diagnosis in other cause mortality. https://doi.org/10.1371/journal.pone.0214860.g004
Source publication
Objective
Extra nodal natural killer/T-cell lymphoma (ENKTL), nasal type is a rare and highly aggressive type of non-Hodgkin's lymphoma (NHL) commonly presented in the nasal cavity or lymphatic system. However, the common causes of mortality in ENKTL remain unclear. We conducted a retrospective population-based cohort study to elucidate the differe...
Citations
... Natural killer/ T-cell lymphoma almost entirely arises in the non-nodal area, thus termed Extranodal NK-T-cell lymphoma; it is divided into nasal (80%) and extra nasal subtype (20%) based on the origin of derivation. Nasal type derived from the aerodigestive tract, such as nasal and paranasal areas (70%), Waldeyer's ring, oral cavity, larynx and hypopharynx, whereas the extranasal subtype exhibits at the skin (10%), liver, spleen (5%), lung, testis or gastrointestinal tract [1][2][3][4][5]. Early dissemination with involvement of peripheral blood is typically addressed as "aggressive NKcell leukaemia/lymphoma" [5]. ...
Extranodal natural killer T-cell lymphoma, nasal type (ENKTCL), is a rare form of non-Hodgkin lymphoma that is strongly related to Epstein-Barr Virus (EBV) infection and commonly presents as “midline lethal granuloma.” Herein, we report a middle-aged lady who presented with a two-week history of fever, sore throat and constitutional symptoms. Intraoral examination revealed a lacerated soft palate with an ulcerated uvula. A diagnosis of ENKTCL was confirmed through deep biopsies under general anaesthesia supplemented with a positive serum EBV genome. Unfortunately, she succumbed due to disease progression with left frontal brain metastasis with concurrent pulmonary tuberculosis before treatment was completed. The recommended treatment is multimodality with L-asparaginase-containing regimes chemotherapy in an advanced stage, relapsed, or refractory ENKTCL for better outcomes. The quantification of circulating plasma EBV
deoxyribonucleic acid (DNA) is helpful as the baseline of tumour load and a biomarker for monitoring treatment response and prognostication. We advocate repeated and deeper core tissue biopsies
... Neerukonda et al [8] recently reported more cases of secondary than primary lacrimal sac lymphoma. Extranodal NK/T-cell lymphoma, nasal type is a rare and highly aggressive non-Hodgkin's lymphoma (NHL) [20] that is more common in Asia and Central America than in the United States and Europe [21] and is highly associated with EBV infection [6,[21][22] . This malignancy mostly involves the nasal cavity and paranasal sinuses, and more rarely the lacrimal sac and nasolacrimal duct, and the prognosis is worse for patients with lymphoma in the lacrimal sac than in the nose [22] . ...
Aim:
To determine the clinical characteristics, pathological types, tumor markers, treatments, and outcomes of Chinese patients with primary lacrimal sac lymphoma.
Methods:
This case-based retrospective study analyzed 15 Chinese patients with primary lacrimal sac lymphoma. The clinical data collected included gender, age at diagnosis, symptoms, imaging examination results, pathologic diagnosis, pathogen identification, tumor markers, treatments, follow-up, and prognosis. Descriptive statistics were used to characterize the patients. Progression-free survival (PFS) was defined as the time from surgery to the last follow-up, first record of tumor recurrence, or death.
Results:
There were 7 males and 8 females with unilateral primary lacrimal sac lymphoma in the left eye (n=6) or right eye (n=9). The initial symptom in 13 patients was epiphora, and 2 patients had redness and swelling in the lacrimal sac area. All patients ultimately developed epiphora, and 12 had masses in the lacrimal sac area. Analysis of preoperative plasma tumor markers indicated 14 patients had elevated homocysteine, 9 had elevated β2-microglobulin, and 2 had elevated lactate dehydrogenase (LDH); 2 patients had elevations of all three markers, and 1 patient had no elevation of any marker. All patients underwent surgical resection and 12 patients received postoperative chemotherapy. The pathological types were DLBCL (n=8), MALT lymphoma (n=5), and NK/T-cell lymphoma, nasal type (n=2). The mean follow-up time was 25.8mo (range: 4-41) and 2 patients died. Seven patients who underwent mass excision combined with dacryocystorhinostomy (DCR) had no postoperative epiphora. Eight patients who only underwent mass excision had varying degrees of postoperative epiphora. Preoperative LDH elevation and NK/T-cell lymphoma, nasal type were associated with poor prognoses.
Conclusion:
Early diagnosis and treatment can lead to a good prognosis for most patients with primary lacrimal sac lymphoma. Mass resection combined with DCR can reduce the occurrence of post-surgical epiphora. The pathology type and tumor marker status are associated with prognosis.
... 243 The reported OS rate was approximately 42%, whereas CA: A Cancer Journal for Clinicians the 5-year PFS rate was only 29%. 268 A prognostic index for patients with ENKTCL was developed using retrospective data and is called the prognostic index for natural killer cell lymphoma (PINK). 269 Four independent risk factors are incorporated into PINK: age older than 60 years, stage III/IV disease, distant lymph node involvement, and non-nasal type disease. ...
Sinonasal malignancies make up <5% of all head and neck neoplasms, with an incidence of 0.5–1.0 per 100,000. The outcome of these rare malignancies has been poor, whereas significant progress has been made in the management of other cancers. The objective of the current review was to describe the incidence, causes, presentation, diagnosis, treatment, and recent developments of malignancies of the sinonasal tract. The diagnoses covered in this review included sinonasal undifferentiated carcinoma, sinonasal adenocarcinoma, sinonasal squamous cell carcinoma, and esthesioneuroblastoma, which are exclusive to the sinonasal tract. In addition, the authors covered malignances that are likely to be encountered in the sinonasal tract—primary mucosal melanoma, NUT (nuclear protein of the testis) carcinoma, and extranodal natural killer cell/T‐cell lymphoma. For the purpose of keeping this review as concise and focused as possible, sarcomas and malignancies that can be classified as salivary gland neoplasms were excluded.
... Our results showed a 10-year OS of 62.3% (IE) and 47.1% (IIE) and with only six deaths reported 5-10 years after treatment, which indicates that the 5-year OS could predict the long-term prognosis and that the 'sandwich' method is a promising treatment strategy for the long-term management of ENKTL. The impact of sex differences on the OS of ENKTL was studied using the Surveillance, Epidemiology, and End Results (SEER) data, which showed that female patients had better OS than male patients [10]. Our study conforms to this finding, and the 10-year OS of females in our study was superior to that of males (74.3% vs. 49.2%, ...
... Natural killer/T-cell lymphoma (NKTCL) is a rare and aggressive subtype of NHL originating from NK lymphocyte and cytotoxic T lymphocyte [2] and associated with Epstein-Barr virus infection [3]. NKTCL is endemic in East Asia and Latin America while rare in Europe and South America [4], usually associated with a poor outcome [5]. Due to its atypical early clinical manifestations, including general symptoms such as fever, night sweats, and fatigue, as well as related symptoms involving organs. ...
Background
Natural killer/T-cell lymphoma (NKTCL) is a rare and aggressive subtype of Non-Hodgkin’s Lymphoma. CircRNA has shown great potential to become a biomarker in plasma. In this study, we aimed to determine circRNA for its diagnostic and prognostic value and biological function in NKTCL.
Method
The circRNA microarray of plasma from NKTCL patients and healthy donors were conducted. The relative expressions of target circRNA were verified by qRT-PCR. We conducted function experiments in vitro and in vivo. Bioinformatics predicted the target miRNA of the target circRNA and the binding site was detected by the dual luciferase report assay. Downstream target protein was predicted and detected by western blot in vitro and immunohistochemistry in vivo.
Result
By analyzing the plasma circRNA microarrays in NKTCL, 6137 circRNAs were up-regulated and 6190 circRNAs were down-regulated. The relative expressions of circADARB1 were significantly higher in NKTCL patients. The knockdown of circADARB1 inhibited proliferation of NKTCL cells in vitro and in vivo. CircADARB1 could bind to miR-214-3p in the downstream and regulate the expression of p-Stat3. In nude mice tumor tissue, p-Stat3 was under-expressed in the circADARB1 knockdown group.
Conclusion
CircADARB1 was highly expressed in NKTCL plasma and circADARB1 was a potential biomarker to assist diagnosis and predict the response in NKTCL. CircADARB1 bound up to miR-214-3p and regulated p-Stat3.
... 23 In our previous study on extranodal natural killer/T cell lymphoma in the USA based on the SEER program, the most common cause of death was NHL. 24 The mortality rate of cardiovascular disease in those with NHL was 5.35 times that of the general population. 25 A SEER-based study showed that 5.8% of NHL patients died of cardiovascular disease. ...
Purpose
Non-Hodgkin’s lymphoma (NHL) comprises many serious hematologic malignancies from lymphocytes. The incidence of NHL is 5.1 per 100,000, with a mortality rate of 2.5 per 100,000 worldwide. However, the causes of death among patients with NHL vary. This retrospective cohort study aimed to elucidate the primary causes and specific risk factors for NHL.
Patients and Methods
The study included patients who were diagnosed from January 2006 to January 2018. Grouped by sex, Ann Arbor stage, date of diagnosis, age, B symptom, NHL type, international prognostic index, and Eastern Cooperative Oncology Group (ECOG) performance score, the Log-rank test was performed, and survival curves were drawn using the Kaplan–Meier method. The competing-risks regression model was used to analyze the specific causes of death.
Results
T-cell lymphoma, B symptoms and worse performance were associated with a lower survival. Mortality from NHL accounted for most and other common causes that contributed to death included circulatory and respiratory diseases. Patients diagnosed with T-cell lymphoma were more likely to die of NHL rather than other causes. Moreover, patients with B symptoms on admission were more likely to die of diseases of the circulatory system. Lastly, patients diagnosed at an earlier age suffered more from diseases of the digestive system and immune mechanism or other uncommon causes.
Conclusion
Classifications of subtypes, age and occurrence of B symptoms were factors providing references for a specific cause of death owing to NHL, which might enable physicians to decrease cause-specific mortality rates.
Background
Patients with sinonasal malignancy (SNM) present with significant sinonasal quality of life (QOL) impairment. Global sinonasal QOL as measured by the 22‐item Sinonasal Outcomes Test (SNOT‐22) has been shown to improve with treatment. This study aims to characterize SNOT‐22 subdomain outcomes in SNM.
Methods
Patients diagnosed with SNM were prospectively enrolled in a multi‐center patient registry. SNOT‐22 scores were collected at the time of diagnosis and through the post‐treatment period for up to 5 years. Multivariable regression analysis was used to identify drivers of variation in SNOT‐22 subdomains.
Results
Note that 234 patients were reviewed, with a mean follow‐up of 22 months (3 months–64 months). Rhinologic, psychological, and sleep subdomains significantly improved versus baseline (all p < 0.05). Subanalysis of 40 patients with follow‐up at all timepoints showed statistically significant improvement in rhinologic, extra‐nasal, psychological, and sleep subdomains, with minimal clinically important difference met between 2 and 5 years in sleep and psychological subdomains. Adjuvant chemoradiation was associated with worse outcomes in rhinologic (adjusted odds ratio (5.22 [1.69–8.66])), extra‐nasal (2.21 [0.22–4.17]) and ear/facial (5.53 [2.10–8.91]) subdomains. Pterygopalatine fossa involvement was associated with worse outcomes in rhinologic (3.22 [0.54–5.93]) and ear/facial (2.97 [0.32–5.65]) subdomains. Positive margins (5.74 [2.17–9.29]) and surgical approach—combined versus endoscopic (3.41 [0.78–6.05])—were associated with worse psychological outcomes. Adjuvant radiation (2.28 [0.18–4.40]) was associated with worse sleep outcomes.
Conclusions
Sinonasal QOL improvements associated with treatment of SNM are driven by rhinologic, extra‐nasal, psychological, and sleep subdomains.
Extranodal NK/T-cell lymphoma, nasal type (ENKTL-NT) is a rare type of Non-Hodgkin’s lymphoma, which usually presents with extranodal involvement and affects the nasal/upper aerodigestive tract in the classical presentation. Herein, we report the case of a 31-year-old, previously healthy, male patient diagnosed with ENKTL-NT with the involvement of the lung parenchyma and heart. Unfortunately, due to the rapid disease progression, the diagnosis was performed only at the autopsy. The authors highlight the rare clinical presentation of this type of lymphoma, as well as the challenging anatomopathological diagnosis in necrotic samples.
